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What
Is Creutzfeldt-Jakob Disease?

Creutzfeldt-Jakob disease (CJD) is an infectious disease that
causes the brain to degenerate. The hallmark of this disease is mental
deterioration and involuntary muscle spasms. Over time, the disease causes growing
problems with memory, personality changes, and dementia. CJD progresses rapidly
and can be fatal.

What
Are the Symptoms of Creutzfeldt-Jakob Disease?

changes in personality and behavior, which are
more common in variant CJD

confusion or disorientation

convulsions / seizures

muscle twitching and stiffness

sleepiness

difficulty talking

blindness

In variant and classic CJD, dementia causes your mind and body to
deteriorate quickly. This is very different from Alzheimer’s-associated
dementia, which progresses slowly.

What
Causes Creutzfeldt-Jakob Disease?

CJD is caused by an infectious agent called a prion. Prions are a type of small
protein that are normally found in the tissues of many mammals. In prion
disease, these proteins are abnormally folded, and form clumps. When they
infect a mammal, its normal proteins start to take on the incorrect structure of
the infectious prions. This causes brain injury by destroying nerve cells and disrupting
the structure of your brain. CJD is a type of “spongiform” encephalopathy. On
brain imaging, people with CJD appear to have holes in their brains where cells
have died — causing their brains to resemble a sponge.

CJD and Mad Cow Disease

There’s strong evidence that the agent responsible for bovine
spongiform encephalopathy (BSE) in cows, commonly called “mad cow disease,” is
also responsible for one form of CJD in humans, called “variant CJD.”

BSE affected cattle in the United Kingdom, primarily in the 1990s
and early 2000s. Variant CJD (vCJD) first appeared in humans in 1994–1996,
about a decade after people first had extended exposure to potentially
BSE-contaminated beef. According to the Centers for
Disease Control and Prevention, this fits the known incubation periods for
CJD. Experimental studies on mice have also provided evidence supporting the
link between BSE and vCJD.

The
Different Types of Creutzfeldt-Jakob Disease

The two most common types of human CJD are sporadic CJD and
familial CJD.

Sporadic CJD

Sporadic CJD can develop anytime between the ages of 20 and 70.
However, it most commonly affects people in their late 50s. Sporadic CJD has no
connection to mad cow disease.

Sporadic CJD occurs
when normal proteins spontaneously mutate to the abnormal prion type. According
to NINDS, at
least 85 percent of CJD cases are sporadic. Sporadic CJD is most common in
people over the age of 65.

Familial CJD

Inherited CJD accounts
for 5-15% of CJD. It occurs when you inherit a mutated gene associated with
prion disease from a parent. People with inherited CJD often have family
members with the disease. The extent of how CJD manifests in separate family
members can vary widely and is known as variable expressivity.

Variant CJD

Variant CJD or vCJD occurs in both animals and humans. When it
appears in cattle, it’s called mad cow disease, or BSE. According to the Mayo
Clinic, vCJD affects mostly young people in their late 20s.

You can become infected with vCJD by eating meat that’s
contaminated with infectious prions. However, your risk of eating infected meat
is very low. You can also become infected after receiving blood or transplanted
tissues, such as a corne, from an infected donor. The disease can also be
transmitted by surgical instruments that haven’t been properly sterilized.
Fortunately, there is rigorous sterilization protocols for instruments that
have been in contact with tissue at risk for prion exposure, such as brain or
eye tissue.

Despite all the press on mad cow disease, vCJD is very rare. From
October 1996 to March 2011, WHO says there
were only 224 cases reported worldwide. As of 2013, only three cases of variant
CJD have been reported in the United States. Less than 1 percent of people with
CJD have the variant type.

Who
Is at Risk of Creutzfeldt-Jakob Disease?

The risk of classic CJD increases with age. You can’t get CJD
from casual exposure to people who are infected. Instead, you need to be
exposed to infected bodily fluids or tissue.

Caregivers of people with CJD should take extra precautions to
lower their risk of contracting the disease:

Protect your hands and face from exposure to
body fluids.

Make sure to wash your hands, face, and all
exposed skin before smoking, eating, or drinking.

Use waterproof bandages to cover cuts or
bruises.

How
Is Creutzfeldt-Jakob Disease Diagnosed?

To diagnose CJD, your doctor will begin with a complete medical history,
physical examination, and neurological evaluation. The rapid progression of
symptoms distinguishes CJD from other causes of dementia. Your doctor can also
use a number of tests to establish your diagnosis:

MRI

MRI is the most helpful test to diagnose CJD. It can detect small
changes to your brain that may suggest CJD. An MRI uses magnetic fields to
create images of your brain.

CAT Scan of Your Brain

CAT scans not as useful as MRI scans to detect brain changes in
CJD. Usually, the CAT scans will be normal. In some patients, rapid
degeneration of brain tissue can be detected.

Lumbar Puncture (Single-Use Kit)

In this test, your doctor will use a thin needle to puncture the
lining of your spinal cord to obtain spinal fluid. If your spinal fluid tests
positive for elevated levels of a protein called 14-3-3, you may have CJD.
However, high levels of protein are also found in many other diseases.

EEG

In this test, your doctor will use scalp electrodes to examine your
brain waves. If you have CJD, your brain waves may show sharp spikes.

Blood Tests

Your doctor can use blood tests to identify and rule out problems
such hypothyroidism and syphilis, which can also cause dementia.

It is important to remember that only a biopsy of brain tissue
can confirm a diagnosis of CJD.

Can
Creutzfeldt-Jakob Disease Be Treated?

There is no known cure or effective treatment for CJD. However,
medications can be used to treat some of the mental changes and personality
abnormalities that occur. Your course of treatment will probably focus on
making you comfortable and helping you function safely in your environment.

What
Is the Long-Term Outlook?

Sadly, according to NINDS, 90
percent of people with classic CJD die within a year. People with variant CJD
tend to survive a little longer, from the onset of symptoms to death.

Symptoms of CJD will get worse until you lapse into a coma. The
most frequent causes of death for people with CJD are: